"Brain in Congenital Heart Disease..."

There are still so many scary things Job gets to deal with as he ages and it's hard for me to keep from discouragement. But I'm so grateful for the vast amount of research already done and improvements in care already made. And I'm especially excited to see what is on the horizon for these kids as they grow into adulthood.

Reading through literature, both past and current, is one of the more helpful ways for me to see this and stay hopeful.

"Brain in Congenital Heart Disease Across the Lifespan: The Cumulative Burden of Injury" (2016)

Ariane Marelli, Steven P. Miller, Bradley Scott Marino, Angela L. Jefferson, Jane W. Newburger

Oral Aversions and Therapy

Job seems to continue to improve. Yay! He slept for an entire four hour stretch last night!?! Amazing!

He had a good therapy session today (something that we haven't been able to say for the last three weeks). I'm so thankful for the amazing therapists we have! Job lost quite a bit of ground this last month as he's been so sick, but for every two backward steps he takes we're going to keep pushing for a forward step now and then. A new concern that, likely because of tasting so much vomit for so long, Job has developed some oral aversions. This is yet another completely new issue for us to now navigate.

Possible Improvement?

We're surviving and maybe, just maybe, seeing improvement!

I think we're realizing that Frightful just isn't strong enough to make it through a cold or tooth coming in without great trial. It's sad and discouraging to realize this, as well as just plain hard to deal with practically speaking as he needs constant, intense care when so sick.

Sunday, Monday and today have each improved upon the day before so we're hopeful this trend continues and we could have a good weekend and early next week before his cath and hospitalization on the 8th.

It's nice to be back at a place where he can cope for 30ish minutes without being held so we can do things like quickly set up a Christmas tree and such. I'm scared something else will come up between now and his cath but I'm trying to choose hopefulness and trust instead of pessimism and anxiousness.

HLHS Explanation Video

https://youtu.be/gyMq0xhnVMU

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Miserable Again

I think this photo sums up the last two days well. Job is miserable again.

We had five wonderful days and now we're back to almost where we were 3 weeks ago in terms of his fussiness (though vomiting seems to be under control?).

Job is definitely teething but we can't think of anything else that has changed. Maybe he's operating on such slight reserve that even something like teething sends him over the edge of misery?

GI Clinic in Everett!

We got to go on our first family-of-five "adventure" (since The Vomitting began) on Thursday to Gastroenterology Clinic. It wasn't the most thrilling destination but it was really nice to be together and out of the house.

We're looking at a likely diagnosis of gastroparesis, which is where the stomach empties slowly. Too slowly. This, with his severe acid reflux, seems to be the primary reason for the vomitting, but teething and a cold haven't helped.

The two medications Job is on do seem to help (omeprazole as an acid neutralizer and reglan as a motility agent).



We have and will continue to investigate diet, but allergies don't seem to be the issue. Our next step would be an endoscopy if things don't improve in a month. We then also need to consider an NJ tube. I didn't realizing a feeding tube was even on the table and the very idea of moving so far backwards is crushing. (But, you know, so would be continued weight loss.)

We have learned how to manage his care in ways that seem to help, like giving 2oz of milk every 2 hours instead of 5oz every 3+ hours. Since he's both getting less milk and still vomiting we'll likely begin fortifying again for greater caloric density. His weight loss seems to have stopped but we have yet to see significant weight gain again.

We haven't even tried to wean off oxygen nor will we for a while. Likewise many of his therapy goals are on pause.

Job's cath is scheduled for December 8th. It will be at least one night inpatient and I think we're all expecting interventions, which might mean a longer hospital stay.

This isn't where we expected to be two months post-Glenn and it's been hard to realize that emotionally but it's been especially hard to just deal with practically. Harder even than when we had bigger cardiac issues, weirdly enough. But it seems like we're starting to emerge from the worst of it? We're at least in a brief respite and very grateful for it, especially because Job isn't so constantly miserable. He's even happy sometimes! Each day this week has been an improvement on the day before.

GI Issues?

Loonngg appointment today, including a full cardiology consult, an echo, a belly x-ray, full blood panel, and new meds.

We won't hear about the blood draw until tomorrow but everything else checked out ok.

It was gratifying to hear Job's team acknowledge that this isn't isn't right and to have them order such a full work up of tests, even though I would have preferred for them to tell me that I was being paranoid and impatient.

We're mostly sure that it's not heart related, and while we're hoping GI has answers (beginning in our Thursday appointment), we're now exploring liver and pancreas issues as possible culprits.

Job was surprisingly accommodating but of course sad.

Squeaky Wheel Gets an Appointment

Well, I guess I've been enough of a squeaky wheel that we're getting some grease.

Or, put more clearly, Job has a cardiology appointment in Seattle tomorrow morning.

I think (hope?) it's more to placate us than because his cardiologists are so worried, but I appreciate that they listen so well and are so happy to consider our concerns.

Over the last 3.5 weeks Job hasn't gone more than 30 hours without projectile vomiting. He is more consolable but still quite uncomfortable. We have a diagnosis of severe acid reflux, which has been compounded by a cold and by teething the past two weeks. We have a gastroenterology appointment on Thursday in Everett.

Job has had some ok days with some contentment and manageable fussiness and only one or two vomits, but never two in a row. He's eating less and I guess I'm worried about dehydration and just need reassurance that we're not missing something.

Tired and Uncomfortable Still

This photo probably accurately captures where Job is at right now, poor guy. He's slightly more calm and interested in the world again, but still tired and uncomfortable.

He is doing better, but he has a long way to go before he's back to himself. He's sleeping for slightly longer stretches (2ish hours at a time) and he occasionally lets us put him down for a few minutes. He'll even attempt to play with his toys sometimes. He's more consolable; his body is less rigid and tense and he'll snuggle up to us. He's only vomiting twice a day now, on average. These are all improvements.

Wednesday's upper GI scope gave us the diagnosis of "severe acid reflux". Thankfully, it seems as if reglan, which we started Monday morning, is doing it's trick, but we have a full gastrointerology work up next week. This is a totally new area to us and so I have all sorts of questions.

I assumed he had a cold since the big boys and I got sick last week/through the weekend, but he didn't have obvious symptoms like we had... but we weren't sure. But by Tuesday evening it seemed as if the balance was shifting from (what we know know was) agonizing reflux pain to common cold discomfort with more teething pain for good measure. That's still where we are now. His pediatrician checked his ears today, but there's only a bit of redness, no obvious infection. Job's down to 79% oxygen saturation on 1 lpm of flow (which is a decrease of 6-7 points from where he was last week, after we turned him up to a full 1 lpm), so this cold is certainly affecting his respiratory status. (But YAY for having oxygen and not being in the hospital!)

Sadly, his cath is pushed out 4 weeks from today because of this cold. We knew that was likely and of course we understand and agree that we don't want to mess with anesthesia and ventilation when he's sick/recovering from a cold. But it is disappointing that he got sick at all (why can't we keep germs at bay?) and that we have to wait to know the full extent of his cardiac situation. I'm also very sad that Job can't travel and needs me to stay with him through the weekend, so I'll have to miss my grandpa's funeral in CA this Saturday.

But we're grateful for small improvements! Job isn't constantly writhing in pain any more and we have some goals to work towards now. We've been able to get more sleep too. It's still hard to find contentment and joy this week, but we're working on it. God has been very gracious to us this week and we want to be grateful. Certainly we can (and have!) acknowledge(d) the hard things, but we don't want to wallow there.

A Bit of Sleep!

Guess who slept the best they have all week last night??? Well, what three persons actually!?!!

Now Job's off to spend the day at the hospital with Daddy for all sorts of fun assessments to see if we can start getting some answers about this vomit issue.

Awful Week

Quite frankly, this has been an awful week.

Job has been ridiculously inconsolable. He vomits 2+ times a day and has a cold, but it's his irritability that is probably most challenging and exhausting (physically and emotionally).

I was so discouraged, but by so many means and people God sustained us for yet another day. I'm trusting that He will through the night as well.

I've posted this song before, but it has been on repeat this past year and certainly was today:

"Your plans are still to prosper
You have not forgotten us
You're with us in the fire and the flood
You're faithful forever
Perfect in love
You are sovereign over us
Even what the enemy means for evil
You turn it for our good
You turn it for our good and for Your glory
Even in the valley, You are faithful
You're working for our good
You're working for our good and for Your glory"

https://youtu.be/B66DHRY1ITs

Hard Stuff

When I look at this picture I realize how far Job has come, thanks to his physical therapist and special ed teacher. He has gained so much core strength and spatial awareness! I think we have the two best therapists we could have asked for! They teach us so much every week and Job is making huge strides developmentally.

The last week has been rough as we've all struggled through a cold. The kids are slowly getting better! I'm grateful that we have oxygen so that Job could stay home instead of go up to the hospital.

His vomiting continues, despite new medications. I was so hoping they would work! I'll find out Monday morning if we can continue with the GI scope this week or if it will have to be postponed like the cath because of this cold.

Job hasn't slept through the night well since our last discharge, but his cold has exacerbated his sleep issues. Seth especially has taken on the brunt of Job's irritability at night, but between the two of us we're up 6-8 times a night with Job. We're both so nervous about his vomiting, so every cough and choking noise sends one of us (usually Seth) jumping out of bed.

As with everything, we're supposed to divide Job's actual age in half because of his condition and the invasive nature of his surgeries, so when I was asking about Job's (lack of) sleep habits this week I was told to think of him like a 3.5-4 month old baby. I suppose that helps a bit, at least to manage our expectations, but Job was such a fantastic sleeper until the Glenn that it's hard to adjust. We're tired!

To end on a positive note, we had our best week of school ever! The big boys are finally settling into the routine we've been trying to establish since coming home and I'm absolutely thrilled. I would love for us to have good health and good sleep and good school, but since we apparently only get one of those things at a time, I'll take school!

Cardiology Clinic (6 weeks Glenn post-op)

Job had his big cardiology clinic appointment yesterday. I had been counting down the days until this appointment with a great deal of trepidation, along with a bit of desperation (largely because of his frequent emesis).

I think the bottom line report is mostly good, but there is just so much involved in Job's care that I need to write out my report of what all we talked about in order to process it. Want to read along?

Oxygen
Let's just start with the most mortifying piece of information: for the last 2.5 weeks, while using the concentrator at home, I have only given Job 1/10 of the oxygen he was prescribed (but while out and about on his portable tanks he's received his full amount of 1 lpm). I am horrified and embarrassed and feel utterly irresponsible. I guess we must have played around with the settings during the training and the concentrator was set on .1 lpm for some example... But came home that way too. I did notice that it said .1 instead of 1, but assumed that the flow meter used a different unit measurement and .1 of whatever this unit was translated to 1 liter of flow. Since we now use the (otherwise unfamiliar) metric system for all of Job's medical measurements, I often have to clarify his meds with various providers as they often use different units. One doctor will talk in mgs while another will talk in mcgs and so on. When clarifying these med doses, I always feel silly (I am still insecure about my lack of mathematical confidence, after all) but have done it anyway to make sure. And I guess this time I just gave up clarifying. Perhaps the lesson is that unit conversions in Saxon Math homework really is helpful later on in life.

So Job's sats have slowly climbed from 76% to ~81% while on "1" (really .1) liter of oxygen, but this very slow rate of improvement to a mere 81% had us all worried. But last night as we decided to put Job down to .5 lpm on his oxygen, Seth - not me - went to change the flow meter. And he couldn't figure out how to go down lower that where I had it set... As the realization of what I had done set in, we put Job up to .5 lpm from .1 and immediately his sats rose to 86%. Today in clinic he was reading at 89% on .5 lpm.

Job's cardiology team just laughed at (with? maybe someday...) me for my mistake, since the whole time at home Job hadn't ever dipped below 75% O2 sats (his cut off). They probably laughed in great relief too, now that we had an answer for his "lack" of *acceptable* improvement.

It's now up to us to play with his oxygen wean as we see fit. For these next few days we'll probably keep him at .5 lpm most of the time and if he continues to thrive we'll probably keep him on .5 lpm during the night for a bit after that.

We're going to keep our tanks and the concentrator for the foreseeable future in case of a cold or emergency. It is nice knowing we have oxygen available so that if his sats start declining we can out him on oxygen at home instead of take him to the hospital (if that was the only reason to admit).

We'll notice a need for more oxygen if his work of breathing increases or he gets particularly cyanotic (blue) but mostly through regular pule ox checks.

Vocal Fold
Job passed his PT swallow check and no longer needs to be on thickened feeds! Yay!

We will almost certainly have to do another scope with Otolaryngology but their wait list is so long it will probably be a few months out.

His vocalizations and cry are still very quiet and raspy so there was almost certainly additional injury to his vocal cord during his arch repair. We originally thought his noticeable inflammation and raspy voice was just because of intubation and reintubation but if that were all it would have healed more significantly than it has. So we will look for continued but gradual improvement over the next six to eight months. It's entirely possible Job will always have a raspy voice because of this, but eventually his vocal cord should close fully (and then reduce his risk for aspiration).

Vomiting
This is the area where we had/have the most concern. Job is vomiting a moderate to large amount every day without a clear reason every time. Sometimes it is because he chokes himself with his hands. Sometimes it is because he coughs up his meds. Sometimes it is because he's still struggling to regulate his digestion and when he strains to poop he vomits because of the effort. Sometimes it is because he got really, really upset (like during today's echo).

Job is drinking so much (~900 mLs/day) and gaining great weight (~20 grams/day) that we aren't yet worried because of dehydration or nutrition concerns.

But it is a very new problem and is currently inexplicable.

So the plan is that we're going to start with a stool softener because his emesis does seem most tied to his bowel regularity, but after a few days we'll also go on reglan. And next week we'll add Gastroenterology to our every growing list of clinic followings and do an upper GI tract scope.

Echo Findings
I pushed for an echo because we're so concerned about Job's inexplicable vomiting. His liver was a bit swollen when palpatated, plus he's Job and has quite the reputation, so I think that makes it much easier to get oil on my squeaky wheel questions.

Good news first: Job's heart function looks good. Yay! His tricuspid valve regurgitation looks the same.

Not so good news: the flow out of his aortic arch seems too fast and there appears to be narrowing in his left pulmonary artery where the stent was placed. Echos cannot accurately show the severity or significance of these issues so it is possible that both of these concerns aren't really concerns at all. But they were alarming enough that we will be getting a cath in about two weeks.

Dr. Chan's best guess, based off of today's echo, was that we will likely need to balloon the stent. As with every cath, Job will be intubated and sedated, but if it's purely diagnostic and no interventions need to be made then he could go home the same day. If interventions are made then he would spend at least one night inpatient for observation. To need to intervene so soon after the Glenn would be very concerning and, of course, a cath carries some risk (perhaps most especially for clotting), but of course we need to know what precisely is happening with Job's heart and lungs, so a cath it is!

Hematology
Our hematology consult was mostly uneventful and unexciting. We were basically just establishing care with them as yet another clinic following Job. They agreed with the cardiology recommendation that we follow a six month regimen of lovenox for Job because he is so hypercoaguable (a standard course is eight weeks). They also recommended that we plan on starting a heparin drip before he has any additional central lines placed in the future. Most of his clots are due to these lines so we want to be proactive about them from here on out. (And they also recommended that he never smoke as smoking will drastically increase his clot risk! I mention this just because it's bith funny and fascinating to me. It's hard to imagine my baby growing up and smoking of all things and it's amazing to realize how invasive the health risks from smoking are.)

We will draw for his Antithrombin III levels at our next lovenox level draw because we need to see what his normal Antithrombin III levels are. I don't think I ever mentioned it but Job needed a dose of thrombate while in the CICU in one of his multiple transfusions. If his levels are still low this could be one of the pieces for why he clots so easily but probably wouldn't be the only reason.


Whew! It's a lot to take in, as always. Sometimes I leave Seattle Children's with my head just spinning with information. I'm so grateful for the incredible level of care we receive there! And as overwhelming as it feels to have November suddenly fill with ten more medical appointments, I'm so grateful Job's team is so vigilant.

Prayer Warriors!

Job got to meet one of his most faithful prayer warriors today.

I suppose introducing Job to Titus was especially sweet, not just because of Titus' enthusiasm, but because it was yet another reminder of how faithfully our family has been prayed for these past months. There are so many people (some that we don't even know!) who remember us in prayer daily and we are so very grateful!

Emesis

This little guy sure loves his food tastings and his Daddy! He has such a joyful attitude, even when he doesn't feel well, like this evening.

Wasn't I just writing about the fragility of life? The mountains and valleys of life with a CHD? Job's health feels so tenuous. It seems as if for every forward step he takes, there is at least one or two backward steps. His sats have been holding steady at 80-81. He even hit 82 this morning and there was talk of trying to go down to a half liter of oxygen.



But... He has vomited eight times in the last 44 hours. And we're trying not to get too worried. Yet.

As always, he has a whole team of doctors watching him closely. We're all on high alert but there's no apparent reason for these "emesis episodes" (as I have to chart them in the tablet, which makes me laugh).

My mind immediately jumps to scary things like heart failure because poor gut profusion is one of the red flags for heart issues (the gut can do without blood flow in crisis better than, say, the brain). I KNOW that's an overreaction at present but it doesn't FEEL like one.

I always struggle with conclusions for these updates. Job's health has never rested in our hands and that hasn't changed these last couple of days.

NICU Follow Up (6 months)

Job had a his big 6 month developmental assessment yesterday (10/24).

As we were waiting for one of the several providers to come in to see us, I snapped this photo of him playing with his feet. We've been working on developing this skill for the last week and a half with several exercises from Job's wonderful physical therapist, but this was the first time I'd ever seen Job do it on his own!!



Job is quite behind on his physical developmental milestones and gross motor skills, scoring around 3 months of age (though he's 6 months old). Perhaps especially because of this, the team of therapists yesterday was quite surprised and pleased with his social and emotional responsiveness scores, which were much more on target for his age.

Job's sats aren't really improving and we're starting to get nervous about what this could mean. It's probably not helpful to even think about right now because he is still allowed more time to get them up, but it's hard to fight the temptation to anxiously worry about his sats and all of the potenial reasons he's satting this low compared to where we want him to be (and where he needs to be to get off oxygen).

If it weren't for his inadequate sats we'd probably feel a much greater sense of relief to be post-Glenn and able to switch our focus more fully to therapy. Right now it feels like we're in an awkward waiting phase.

Honestly, in some respects I don't know if we ever will be out of a waiting stage. Isn't that what life on earth is? But with his diagnosis, Job's life will always be particularly fragile and we will likely have many complications and scares. Sometimes I forget that, especially as he is improving and, it seems, entering a more stable stage of life. I suppose we will eventually find a balance!

One Week at Home!!

Job has been home from the hospital for one whole week!

The week has flown by as it's been a busy week of appointments for Job! I kept telling myself that, once we got through Thursday, things would slow down. But Monday it starts up again! Physical therapy, pediatrician well child, blood draw, early childhood intervention therapy and cardiology appointments... It takes lots and lots of people checking on Job to keep him healthy!

We were doing a very slow narcotic wean but, perhaps since he was also teething, we realized it wasn't slow enough. Taking a few days off from the wean was so nice! But we started back up yesterday and have about a week left.



Job's sats just aren't high enough. Still. Even on 1lpm of oxygen. He had a long cardiology appointment today, here in Tacoma, and everything that they can see by echo looks good. The thought continues to be that his low sats are pulmonary embolism or pulmonary lung bed related and will, hopefully, resolve with more time.

We have an appointment in Seattle on November 1st and, if his sats aren't improved by then, talks about a cardiac cath will begin.

Therapy and our subsequent practice sessions exhaust Job, but we'll keep working on his endurance! It's so exciting to watch him learn new skills. It's very hopeful to see progress day by day and week by week. I'm learning so much about how to help his little body learn to move!

He's doing a great job tracking our voices or toys and reaching out (even across his body!) for objects and he has great head and neck control. He loves sitting up on our laps, watching the world around him. As of this week he has been sitting in a bumbo (pictured) and his high chair, but working his core like that is particularly exhausting! He can roll to his side but until his sternal precautions are lifted next week we can't work on tummy time or putting any weight on his chest and arms. I'm excited for him to eventually learn to roll over! That might be his next milestone.

We reduced the fortification of Job's milk in the hospital, but even with fewer calories per bottle Job is gaining about 9 grams a day. Yay! We want to see that number a bit higher (15 grams!) as weight gain will help strengthen his body and lungs in particular (which ought to help his sats).

We're trying to relearn living together as a family and trying to catch up on the last five weeks of mostly-incomplete school work. It's a big adjustment! I think we're also trying to recover from the emotional exhaustion of what proved to be a rather traumatic hospital stay. And we're trying to avoid any and all cold and flu germs!!

We are all so happy to be together again. There has been an awful lot of baby snuggling this week.

Thankful

Thankful for Tylenol, to break through difficult opiate weans.

Thankful for big brothers, who share their snakes.

Thankful for happy smiles, the persistence of joy through pain.

HOME

It is so good to have Frightful home. So so good.

Leaving the hospital was a catalyst, I suppose, to think about everything that happened while we were there. Including how very close we were to losing Job. I guess now that he's stable and home I've been thinking (and dreaming) about how critical he was. We are so grateful Job is alive! But it's going to take a while to recover from this hospitalization.

Job has moments of joy and contentment, particularly when he's sitting up and watching his brothers play or entertain him. But it's been a pretty difficult last couple of days. He's miserable withdrawing from his dilaudid and clonidine. We think he's teething too. He has 10 more days of this wean as scheduled, but I may ask to prolong it, depending on how today and tomorrow go.



It's also strange to get used to his oxygen tanks and concentrator. We had to go to the pediatrician and grocery store yesterday and it was hard to remember we were connected to the tank! Ezra and Isaac did a good job of reminding me though.

Actually, Ezra and Isaac have been amazing helpers!! Isaac can get the biggest smile out of his little brother. And Ezra has made it possible for me to draw up meds or go to the bathroom or make food or just have a few minutes break because he can so competently care for his brother and soothe him. How did I ever take care of a baby before without little assistants?? They love their baby brother so fiercely.

HOMECOMING

Guess what my favorite word is? (Hint: it starts with "D" and is 9 letters long.)



We made it!!

At Home Oxygen and Med Schedule

We're getting closer and closer to discharge!



This is our at-home oxygen set up!

We have two portable tanks, one with a stand and wheels and one with a backpack. These tanks don't last very long: 2-8 hrs, depending on what his liter per minute (lpm) flow is. We're planning on 1lpm but may get to wean down eventually.

The blue box is his concentrator which plugs into the wall and takes the room air and filters it, taking out the nitrogen and other gases, delivering only oxygen to Job's body.

When on the concentrator, at least, Job will have a 21 ft tube that connects from the concentrator to his nasal cannula, which has a 7 ft tube. It's on wheels but heavy.

Job will have meds every six hours. It's exciting to be down to only 8 meds, 4 times a day!

Cardiac:
Sildenafil
Enalapril

Anti-coagulent:
Lovenox (for blood clot treatment)

Sedation/pain:
Clonidine
Hydromorphone (dilaudid)

Digestive:
Omeprazole

Diuretic:
Furosemide (lasix)
Spironolactone

Discharge This Week!!?!

Job, by so many measures, is steadily improving. Yay yay yay!!!

His oxygen saturation percentage is wholly insufficient for a post-Glenn baby, but the hope is that, with time, this will improve. The current idea is that the clots in his lungs (discovered in his cath on 9/21) are the culprit for his poor saturation and as they resolve his numbers will improve. (Again, he should be mid 80s, not 79-81.)

If his sats don't improve within a month, he'll need a cath to explain why and future possible interventions.

His opiate wean is going well. His bottle feeds (and weight gain) are going well.

The sad news is that he'll be on oxygen for at least several weeks because of his poor sats.

However, there's good good good news too! Job has been cleared to go home on oxygen!!! Likely this week!!!

JOB COULD COME *HOME* THIS WEEK!!! HOME! As in, LEAVE the hospital!!!

Now, as always, this could change. Also, being on oxygen at home won't be fun or easy. But to be AT home??? I can hardly believe it. But I couldn't be more excited.

Exactly when we go home will depend on how Job continues to do this week and when we can get our Home Care oxygen teaching completed as well as any other scheduling sorts of things.

Sunday Night Update (10/9/16)

Job is getting back to his cheerful little self! He just loves interacting with his brothers and sitting up, watching everyone and playing with his toys.

But (did you hear that coming?) his sats (even on oxygen!) are not adequate. Post-Glenn he should sat in the mid 80s. He's in the high 70s.

Time might be the answer. That's certainly what we're all hoping! We know Job is so slow to respond. Maybe his heart and lungs are still gaining strength.

We put him back on spirnolactone, one of his diuretics, to work on his fluid balance because it is true that his sats have declined this week as he's become more fluid positive. Whether or not there's a correlation remains to be seen.

But it seems increasingly likely that we're headed for a cardiac cath early to mid next week if we don't see improvement within the next 24-48 hours.

I guess I'm glad we're at the hospital for a good week anyway for his opiate wean (which is going surprisingly well! but very slow) because it feels like we have plenty of time since we'd be here anyway, even if he was satting at 85.

Buuuttt it's certainly disappointing as we were so hopeful for the Glenn to be more immediately effective.

I'm trying not to be pessimistic... but also trying to be realistic. I would love for Job to suddenly pull his sats up! But I want to be prepared if that doesn't happen without additional intervention.

It does help seeing Job's happy smiles and I'm thankful that we're seeing our baby return to us, gradual as it may be.

Bottle Feeding!! Happiness!

This is Job's third 5oz bottle of the day. There's talk of pulling the NG tube tomorrow or the next day.

Here's a video of Job from last night. Doesn't he look like a different kid?

The short update is that Job is doing really, really well. He has suddenly made very quick progress and we're still in a bit of shock.

We're planning on about 2ish weeks in the hospital still, primarily for his opiate wean.

Update (10/6/16)

The long update:

Job has so rapidly progressed that my head is still spinning. I can't articulate how unexpected and how (medically) inexplicable and just all around amazing his strides forward have been.

On Thursday morning (9/29) Job was still on 8lpm of oxygen support on hi-flow. Yesterday and today he has had several long breaks off all oxygen!

I wasn't at the hospital quite as much this weekend/week because Seth and Ezra were out of town and Isaac could only be asked to stay at the hospital for so long each day (and he can't sleep here). I think that contributes to my feeling of shock and awe because these changes seem even more sudden.

I went home Saturday night (10/1) feeling cautiously hopeful for the first time in two weeks. Job had made several weans on his respiratory support and narcotic doses and his clot looked stable. But he had been so very critical for so long and his team has been so worried, that I was afraid his weans wouldn't stick but the fact that he had weaned at all and seemed to be turning a corner was exciting. He has shown us so many times, however, that we need to carefully temper our expectations.

So when I came back to the hospital Sunday (10/2), I was conpletely astounded that Job was down significantly on his respiratory support. His ND tube was also pulled back from his duodenum to his stomach, as a NG tube.

We had been aggressive with the narcotic wean, so after a fitful night I asked that he get a break from weaning further on Monday (10/3) - even though he didn't score too high on the withdrawal standard. Since he was already at 2lpm and only 35% FiO2, we also decided to get an x-ray to check his lungs, since he seemed so prone to atelectasis. The attending who came on service that morning kept talking about transferring down to the floor but I didn't really believe her. Until I left for the day to take Isaac to co-op and teach piano and got a call mid-afternoon that he was heading to the step-down ward!

On Tuesday (10/4), Job switched from hi-flow to "wall oxygen" with a regular nasal cannula at lpm but 100% oxygen and stayed steady on that throughout the day and half of Wednesday. He kept oversatting in the 90s, so around noon we decided to just turn it off entirely. He did really really well! But my skepticism it wouldn't last through the night was well founded and he had to go back on around 10pm. He struggled to maintain his sats on 1.5lpm this morning but now he's off it again. We're not entirely sure why. We'll see how the rest of the day goes. We have plenty of time to wean down still.

The very best news is that today's echo seems to show that his atrial clot is gone. G-O-N-E. There's some residual thickness on the atrial wall and it's possible that the clot could have dislodged, but it sounds like his body would have already shown us where the clot went. His neurological, renal and cardiac function seems unchanged and so we're hopeful that, in fact, the clot has been reabsorbed and isn't lingering in the body somewhere, ready to cause problems elsewhere. I've asked three different doctors to look at the images and I still need to hear it from Job's primary cardiologist, but this is such thrilling news.

I was going to ask that Job get to bottle feed yesterday, but my favorite OT/PT was off so we're going to wait until today to work on that. Job doesn't recover from surgery well. He doesn't NOT clot well. But he has always fed well and I'm excited for him to give bottle feeds a try after a break of almost three weeks.

I know many people are excited about Job's progress and are expecting discharge soon. It's still going to be a while, though. Actually, yesterday one of the first year residents came in to talk to me and when I told him Job had been off of oxygen for a few hours he said "well, that means discharge tomorrow!" I laughed (and then immediately felt remorseful for the tone of mockery in my laughter) and asked for him to go confirm that with the attending. Sure enough, no. Job will not be discharging today.

He could go home on oxygen. He could go home on the NG tube. He will go home on anti-coagulants and cardiac meds. So by all of these measures (respiratory, digestive, cardiac) he could be discharged. However, he has to stay in the hospital for his opiate wean. He has seven step down doses to get through before he can discharge and at best we could only do one a day. Realistically we probably need to go every other day. He started off on such a high high dose of dilaudid and it's going to take a while to get it down. Watching my three month old baby go through narcotic withdrawals was pretty difficult the first time around and I'm not looking forward to watching my almost six month old baby go through it again.

So. Job is doing much much better. He still has about 1.5-2 weeks left in the hospital, but compared to how much longer we thought he would have, based on how critical he was? 2 weeks sounds like a quick stay. It's only now that Job is doing better that I'm gaining perspective on how much life support he was on and how very dire his situation was. We certainly knew his doctors were concerned, very concerned, but we didn't have the context to know what that really meant at the time.

We are so very grateful for Job's life, especially as we know how close we were to losing it. That he is now progressing at all, let alone so quickly, is a testament of the Lord's kindness to us.

Wednesday Night Update (10/5/16)

Job has so rapidly progressed that my head is still spinning. I can't articulate how unexpected and how (medically) inexplicable and just all around amazing his strides forward have been.

On Thursday morning (9/29) Job was still on 8lpm of oxygen support on hi-flow. As of tonight, he has been off all oxygen support for 8 hours and he's satting in the low to mid 80s.

I haven't been at the hospital quite as much this weekend/week because Seth and Ezra were out of town and Isaac could only be asked to stay at the hospital for so long each day (and he can't sleep here). I think that contributes to my feeling of shock and awe because these changes seem even more sudden.

 

I went home Saturday night (10/1) feeling cautiously hopeful for the first time in two weeks. Job had made several weans on his respiratory support and narcotic doses and his clot looked stable. But he had been so very critical for so long and his team has been so worried, that I was afraid his weans wouldn't stick but the fact that he had weaned at all and seemed to be turning a corner was exciting. He has shown us so many times, however, that we need to carefully temper our expectations.

So when I came back to the hospital Sunday (10/2), I was conpletely astounded that Job was down significantly on his respiratory support. His ND tube was also pulled back from his duwadnum to his stomach, as a NG tube.

We had been aggressive with the narcotic wean, so after a fitful night I asked that he get a break from weaning further on Monday (10/3) - even though he didn't score too high on the withdrawal standard. Since he was already at 2lpm and only 35% FiO2, we also decided to get an x-ray to check his lungs, since he seemed so prone to atelectasis. The attending who came on service that morning kept talking about transferring down to the floor but I didn't really believe her. Until I left for the day to take Isaac to co-op and teach piano and got a call mid-afternoon that he was heading to the step-down ward!

On Tuesday (10/4), Job switched from hi-flow to "wall oxygen" with a regular nasal cannula at lpm but 100% oxygen and stayed steady on that throughout the day and half of Wednesday. He kept oversatting in the 90s, so around noon we decided to just turn it off entirely. And he's done so well all day!! I took the cannula off around 6pm and he's still holding steady. I'm skeptical it will last through the night because I'm always skeptical about Job's progress.

The very best news is that today's echo seems to show that his atrial clot is gone. G-O-N-E. There's some residual thickness on the atrial wall and it's possible that the clot could have dislodged, but it sounds like his body would have already shown us where the clot went. His neurological, renal and cardiac function seems unchanged and so we're hopeful that, in fact, the clot has been reabsorbed and isn't lingering in the body somewhere, ready to cause problems elsewhere. I've asked three different doctors to look at the images and I still need to hear it from Job's primary cardiologist, but this is such thrilling news.

I was going to ask that Job get to bottle feed today, but my favorite OT/PT was off today so we're going to wait until tomorrow to work on that. Job doesn't recover from surgery well. He doesn't NOT clot well. But he has always fed well and I'm excited for him to give bottle feeds a try after a break of almost three weeks.

I know many people are excited about Job's progress and are expecting discharge soon. It's still going to be a while, though. Actually, today one of the first year residents came in to talk to me and when I told him Job had been off of oxygen for a few hours he said "well, that means discharge tomorrow!" I laughed (and then immediately felt remorseful for the tone of mockery in my laughter) and asked for him to go confirm that with the attending. Sure enough, no. Job will not be discharging tomorrow.

He could go home on oxygen. He could go home on the NG tube. He will go home on anti-coagulants and cardiac meds. So by all of these measures (respiratory, digestive, cardiac) he could be discharged. However, he has to stay in the hospital for his opiate wean. He has seven step down doses to get through before he can discharge and at best we could only do one a day. Realistically we probably need to go every other day. He started off on such a high high dose of dilaudid and it's going to take a while to get it down. Watching my three month old baby go through narcotic withdrawals was pretty difficult the first time around and I'm not looking forward to watching my almost six month old baby go through it again.

So. Job is doing much much better. He still has about 1.5-2 weeks left in the hospital, but compared to how much longer we thought he would have, based on how critical he was? 2 weeks sounds like a quick stay. It's only now that Job is doing better that I'm gaining perspective on how much life support he was on and how very dire his situation was. We certainly knew his doctors were concerned, very concerned, but we didn't have the context to know what that really meant at the time.

We are so very grateful for Job's life, especially as we know how close we were to losing it. That he is now progressing at all, let alone so quickly, is a great answer to a great deal of desperate prayer.

Tuesday Night Update (10/4/16)

Frightful likes his new room in River 6, the step down ward.

To go from such robust ICU support down to a mere 1lpm of oxygen in 4 days has shocked us all. I'm still not sure it's real. (But discharge is still a ways away.)

Tomorrow we push for bottle feeds.

EXCITING NEWS (10/3/16)

https://www.youtube.com/watch?v=BgIuXGK6RZo&feature=share

Saturday Update (10/1/16)

Today's plan: constant cuddling (and the three year old will totally allow that, right?).



Job has been tolerating his narcotics weens pretty well, alternating clonadine and dilaudid weens every other day. So far he has yet to experience his harsh withdrawals but I expect that will happen soon when we get to lower doses.

He is satting in the 80s even when upset. IN THE 80s!!!! And he's down to 5lpm, 35% FiO2 on his CPAP. It's amazing. I'm still in shock. He hasn't held on to sats in the 80s since early July.

The plan is to pull his PICC line out of his heart today, essentially making it just an IV line instead of a central line. I want it gone asap because I am now so nervous about him clotting. But it has been really nice to have the access for blood draws instead of pokes each time. With this we'll switch to lovenox injections instead of the heparin drip. They're still having a hard time getting his levels stable so he'll keep the line until they are able to get his dosage stable because until then he needs blood draws every 4-6 hours to check levels.

I'm starting to let myself feel hopeful again. But I'm scared to too, because historically every we start to get excited about his progress is when something else goes wrong (and for the last 5 weeks, the new bad news always hits Monday afternoon).

Life with Job is a roller coaster of emotions! But we'll never be bored?

Thursday Update (9/27/16)

How's this for cozy?

Job's morning echocardiogram again showed no clot growth or movement. I think everyone is slowly starting to breathe a bit easier. Each day that passes lessens the likelihood of growth or movement, although there will certainly be risk until the clot is (hopefully) reabsorbed.



It seems definite that Job will be on an anti-coagulant for the rest of his life. He's on a heparin drip through his PICC now and will be switching over to lovenox (which is actually a form of heparin) injections this afternoon. And in a few weeks when we, Lord willing, go home, he'll switch to an oral dosing (like warfarin). Baby aspirin just won't be enough for him as his clot factor is too high.

He's down to 7lpm on his hi-flow and seems to be doing well. If his sats drop or his work of breathing increases then he'll go up to 8lpm of pressure again or even back to BiPap.

Isaac is very happy to entertain his little brother and Job is very happy to be entertained by his big brother. Job is responding more and more to us, slowly resembling the little baby that we used to know.

Wednesday Update (9/28/16)

This is Job's current vascular map, showing where he has/had clots. Hematology is still doing their work up. He'll get another echo tomorrow. So far there is no movement or growth, which is hopeful.



Surgery doesn't seem to be a good option. The risk of surgery itself, post-op complications and the likelihood of "stirring things up" and creating new clots all present too high of risks to make it worth it for this size of clot.

Job is having a good day from a respiratory standpoint. He's been off BiPap for 16 hours and doing well. He's at 35% FiO2 (oxygen) and 8lpm of "pressure," delivering that oxygen on hi-flow. Perhaps tomorrow we'll try 6lpm.

Tuesday Update (9/27/16)

Job had very sad morning. He was NPO (no food by tube/mouth) so his tummy felt empty (even though he was on his D5 fluids - sugars and lipids) and he did not like it. He also got lots of blood draws and echos and exams so he was irritated because of those as well.

His lungs look much better and he's tolerating his respiratory support weans so he got an hour off BiPap and was on hi-flow instead. He switched away from the BiPap mask to a nasal mask because, as you can see, the mask was hard on his skin. He will get more and more breaks off of BiPap and on hi-flow if he keeps his sats up and his x-rays continue to show his lungs remain open.

He needed his LPA stent to get greater blood flow to his left lung but his recent echos show that his lung issues no longer seem to be plumbing issues. His team is hopeful that his heart function and pulmonary vascular flow will continue to improve with more time. There does not seem to be an arch issue as was originally speculated or anything else that needs a surgical fix. Job "just" needs more time.



He an echo earlier today to look at his atrial clot but official results aren't back yet. Unofficially, it doesn't look bigger. Part of the clot has adhered to the atrial wall but part of it looks "floppy". The concern is that this floppy part could dislodge and cause great problems. While a clot could be problematic for anyone, the body has an amazing clot filter in the lungs. For typically developed hearts, blood is pumped through the atrium to ventricle to the body and then returns through the lungs to begin the process again. For Job, with his single ventricle heart, there is no filtering process. So his lungs can't catch a traveling clot.

The risk today is not greater than it was last night. But having a slightly better understanding of the situation has certainly impressed upon me the gravity of the concern. This is scary.

His heparin regimen is not to break up the clot but to prevent it from growing and to organize it, to make it tighter, so that the body can resolve it. We want his clot to stabilize and then be reabsorbed by his body. We certainly do not want vein/artery occlusion or stroke or any such clot complications.

Job's body has shown us that its stress response is to clot rather than bleed out. This clot is very likely because of his RA lines, a clot forming and sticking to the intrusive plastic of the lines. We think it is a consequence of how much support he was/is on rather than a symptom, showing us he's getting sicker.

Job went to Interventional Radiology this afternoon for placement of his PICC line. Job is always a hard poke for lines and he goes through his PIVs quickly, so despite the increased risk of clot at the PICC line site, he needs the more stable access it will provide to give him his meds and for the greater ease of blood draws.

After nine long days of being unable to hold Job, we were finally able to Sunday night. He likes being held but we probably enjoy it even more than he does.

I don't really know how to talk about his condition right now. We're sad, very sad. Concerned too. I know it's a good thing to look for progress and be excited for the progress he has made, but right now it just hurts to hear really happy, positive, hopeful comments. I don't want to minimize progress, but Job's situation is very critical and we're very aware of that.



We know God has a plan for Job that will be for our good and His glory. But it's currently a very painful plan. We are now expecting weekS in the CICU.

Tomorrow will bring another echo and anti-coagulant levels and perhaps additional clarity. He's getting food again, he won't get poked now that he has a PICC line, and we expect he'll get several BiPap nasal mask breaks, so he should be a much happier guy tomorrow.

Monday Update (9/26/16)

Today's echo showed Job now has a blood clot in his atrium. He's on a full therapeutic dose of heparin, a blood thinner. The clot could move but he's somewhat stable right now on heparin. There have been and will be many more conversations between cardiologists and interventionists and surgeons and hematologists but I don't think anything will happen tonight.



Tomorrow's echo will tell us a lot about what our next steps will be. If the clot moves or grows that likely means another open heart surgery to remove the clot.

Job has made (very slow) progress this week. It feels difficult to do, but we want to be excited for him and acknowledge this. Last Monday evening we were very worried about him. Things needed to change and they did change. He has gone down on many meds and some of his respiratory support. His sedation balance is better. His heart function has improved a bit. His sats are better.

But now we have a brand new set of scary, dangerous problems.

Sunday Update (9/25/16)

Over Saturday and Sunday Job experienced so many changes in his care!!



He got his RA lines (central IV lines, directly in his heart), pacing wires and chest tube out today!! He has his art line (right hand) in, for blood draws and constant blood pressure monitoring (although he's been working on pulling it out, so it wouldn't surprise me if he list it any time soon) and his new PIV line (left hand) for his precedex and general access if need be. Doesn't he look practically naked now?

He is now off milrinone (which aided the squeeze of his heart) and taking his lasix and spirnalactone (the two diuretic meds he was on pre-Glenn) and omeprazole (a digestive med he was on pre-Glenn too) and dilaudid and clonidine (narcotics) by ND (feeding) tube! His only drip is a minimal dose of precedex (a "calming," anti-agitation med) and that's really to just keep him from ripping off his scuba mask. I can hardly believe it!

These are all great changes and I'm so happy for him that he got his "chest stuff" out. He will be so much more comfortable now!

He is still on a great, great deal of respiratory support. And his heart-lung balance is certainly insufficient, so he's not getting out of the hospital any time soon, but it's fantastic to remove several annoyances for him (and reduce the risk of infection)!

Friday Update (9/23/16)

From a cardiac and/or respiratory standpoint Job is doing much better. In fact, he's doing so well that perhaps we were a little too aggressive and made too many changes too quickly. This, coupled with restless sleep or little sleep seemingly sent Job into one of the most delirious episodes he's had yet.



When I first walked into the room earlier today Job looked absolutely awful. He was cross-eyed, but his eyes kept rolling back into his head. He couldn't stop flailing his arms and legs, but his movements were so uncoordinated. He was feverish too. It took about two hours to calm him down enough so he could sleep. But thankfully, he finally slept well and eventually woke up in a more lucid state of mind. Making sure he gets good sleep will be a priority this week.

We (I use that term quite loosely) backed off on some of his weans because he showed us he's not ready to be pushed so hard. He did well all day on hi-flow and off of CPAP (yay!!). But this evening right before the boys and I left the hospital (siblings are kicked out at 8pm) he randomly desatted in his sleep. He immediately got deep suctioned which didn't help like it usually does, so he got an x-ray to check his lungs.

We're very concerned about his atelectasis (lung collapse) and even though the x-ray looked good (no re-collapse) the decision was made to put him back on CPAP to be extraordinarily careful. He'll probably stay on it all night just to give his lungs the extra support of positive pressure to make sure they stay open and to relieve some of the work of breathing while he's still weak.

I talked to the NP about it for a while because it seemed like a disappointing step back, but we can't push Job too hard. This is how CICU life goes, after all: one step forward, two steps back. An additional factor in the decision was the high acuity on the floor tonight. Job will absolutely get the care he needs, I have no qualms about that! If his nurse were to call for someone they would be there immediately. But giving him the perhaps unnecessary extra support of CPAP tonight will just lessen a bit of the load on the floor tonight as Job will (at least presumably) need less assessment than he would if on hi-flow.

I want to make note of the special blessing it was this week that Job's primary cardiologist, Dr. Files, was the in-service cardiologist in the CICU this week. Of all the possible weeks for him to be on the floor every day, I am so grateful it was this week. He knows Job best (although really there isn't anyone involved with hearts at SCH who doesn't know Job very, very well) and he is such a very kind and wise man. He took special effort to direct Job's care this week and I will always be so grateful.

Likewise, it was simply wonderful that one of our favorite nurses, Alena, was Job's nurse Tuesday through today. She even picked up an extra shift today, surprising me! to care for Job an extra day. I had teased that I'd call a Code Pink (missing persons) on her if she ever left the hospital and then she showed up again today!

I can't think of another nurse who could have been a better fit for Job's/our needs this week. She advocated for Job and loved on him and answered my million questions. Thanks to her careful and oh so patient teaching, I feel like I might finally be starting to understand where Job is at and how some of his meds work. And she even made him a mobile! She had discovered that Job really likes watching the live cam of the Monterrey Bay Aquarium jelly fish tank and shark tank and so last night she found these sea life pictures and cut them out and hung them for him. Right now he's too weak and tired to play with them or look at them much, but it was such a sweet gesture on her part.

There are many such ways that we were specially cared for this week, but those two people were a particular blessing I don't think I've mentioned yet.

If Job has a good day tomorrow I might just left myself start to feel cautiously hopeful about his prognosis!

Thursday Update (9/22/16)

The page for today's updates in ascending order:

8:30am
Can we just forget everything else about Job's health, long-term and short-term, and celebrate this moment?? I could not be more thrilled right now.

Cath #3 Results (9/21/16)

A cardiac catheterization is the best diagnostic tool available to assess cardiac function and the pressures and flows within the heart. An echocardiogram (an ultrasound of the heart) is of course less invasive but can't always give the best assessment. The "cath" is called such because it entails a thin piece of tubing (the catheter) inserted into an artery or vein (in the groin or neck or, apparently, liver). A cath can also be interventional, allowing the ballooning or stenting of a narrowed artery or vein.

Job recieved a LPA (left pulmonary artery) stent (which was described as a loop of fancy chicken wire). This will keep his LPA from further narrowing and it was placed where the green circle is. It's hard to see the narrowing in this particular picture but it was significantly inhibiting the LPA flow. The tissue around the stent will quickly grow around it, keeping it in place, and it won't grow with Job, so it will eventually need dilated (or replaced in a future open heart surgery, such as his Fontan).

Because Job is always one for fun extra surprises, he also had a clot where the purple circle is. It was dispersed with heparin and he will shortly begin a lovenox regimen (and we're old pros at lovenox injections!).

And because we're often asked, the orange circle highlights one of his sternotomy wires, keeping his cracked sternum closed.

Miraculously, Job's aortic arch repair did not need repaired as seemed likely. I think we're still in shock, actually. This is such wonderful news!

Job is not satting as high as we would like, even after this intervention, but it bought him a few days to get his sats up. His heart function likewise needs to improve, but again, he can have a few more days to accomplish that as well.

Surprisingly, Job was extubated in the OR and is on the BiPap at present. Reintubation is still a possibility but if he has a good night then that possibility diminishes. We want him to ween to CPAP then hi-flow then nasal cannula then no respiratory support but, because this is Job we're talking about, it will take some time.

Tomorrow Job will get another ultrasound to look at his diaphragm plication which is possibly coming undone. If this is the case he would get to have surgery tomorrow to fix it, but it is extremely rare for this to be necessary (but again, this is Job... He never takes the easy path).

Unfortunately his plication (which was necessary because of his nerve damage sustained in the Norwood) makes his ability to take deep, full and effective breathes more difficult at present. Around the age of two his thoracic/intercostal musculature will be strong enough that the plication will no longer be such a factor, but it very much inhibits him now. If his phrenic nerve was going to heal, it would have healed by now. So the left side of his diaphragm won't ever be able to move (even if it hasn't been plicated, or stapled down).

Job has yet to fully come out of his anesthesia and is pretty well sedated and will be through the night.

I'm not entirely sure what his goals will be for tomorrow. I think it will largely be a day of rest, to hold steady on the small forward steps he he has made and to assess his goals for the rest of the week.

We are so very relieved that Job has seemingly turned a corner. He was so very sick and had us all extremely worried through Monday. His progress has been tentative but to even use the word "progress" is thrilling. He certainly has a long road ahead of him, but we've done long hospital stays before and we can do one again.

Wednesday Morning Update (9/21/26)

This morning Job was awake, looking at us and listening to us!!! For 15ish minutes! But it wore him out and he's back asleep now.

This means his delirium is largely gone and his sedation balance is being better managed (that sounds like an indictment of his team, but it's not! It's just a hard balance to find).

Job got a NG tube on Sunday and has intermittently taken feeds of 5mL/hour as he's been able to digest them. He's still on his D5 (basically sugars) drip but since things didn't go as expected and he's still sedated and ventilated he can't feed by bottle. And his team didn't want his digestive system to go any longer without being reintroduced to the concept of food. He hasn't always been able to digest the food (depending on how sedated he is) so he's gotten several breaks, but he's done pretty well. But he's NPO (no food by mouth) as of 6am because of his cath today.

He just got his daily x-ray and it showed improvement in his lungs, so we're headed in the right direction there. They need to expand and continue to dry out but should as time passes.

We'll likely spend the morning talking to his team and signing consents and then we get to wait (not anxiously?) for the several hours during his cath this afternoon. Just like during his last cath, we're so desperate for the results of the cath that it's easier to send him off to the cath lab than it might usually be.

We wanted his Glenn to go well! We didn't want to be in the situation, waiting and wondering, but it will be so good to get some answers today.

Seth's Update (9/20/16)

Here are a few quick bullet points about Job's biggest issues/prayer needs:

-The squeeze of his heart (aka "heart function") has not been very strong since the surgery. It seems like the doctors don't exactly know why.

-His Aortic Arch, according to recent echo images, appears to be even more narrow than before the surgery, despite the surgeon's attempts to repair/widen it. If (or when) they do a cardiac catheterization, it will be for the purpose of once again widening his arch.

-His sedation needs, due to his intense pain, must be balanced with his need to breathe deeply on his own. According to Dr. Files, the team must do a better job of managing this balance the next time they attempt a successful extubation.

-His pain and discomfort are causing him to have episodes of severe oxygen desaturation, hence the need for so much sedation/pain meds.

-Autumn and I are pretty shaken up at this point, but Job needs us to be focused, hopeful, worshipful and ready for the day.

In Christ's name, Amen

Update (9/20/16)

Job had a pretty good day today. He is less delirious, more consolable, presumably because his pain is becoming a little more manageable. His sedation/pain medications have been weaned a bit (albeit from very high amounts yesterday). He seemed to have a more peaceful day. He even opened his eyes and looked at us for a few seconds (as opposed to his eyes being rolled back whenever open).

Today's echo images confirmed the narrowing in his aortic arch and his need for a cardiac cath. There are some significant "access" issues, as his right leg is totally blocked by a clot and his left leg is mostly blocked (complications from previous caths). At this point, their first choice of access is through the liver. It will be both exploratory and possibly interventional, perhaps resulting in a balloon or a stent in the arch. We are cautiously hopeful about this procedure. It's a major intervention, filled with risks but with significant potential benefits. He is scheduled for second case tomorrow, which means around 1 pm.

His depressed heart function is still a concern, but could be caused, at least in part, by the narrowed arch. We'll see.

We know that many of you have been praying all day, particularly for our peace and strength. Thank you so much. God's kindnesses have been apparent to us too many times to enumerate.

Today was a small but definitive step in the right direction. Praise God.

-Seth

A Sad Day (9/19/16)

I don't even know where to begin this update or how much to say.

Job is not doing very well. There's probably no other way to say it. But there is still so very much support that can be offered to him to help his body as he struggles. He's not in immediate danger.

We need to give him more time, absolutely. But we're getting to the point where we need to start exploring what is going on with his little body because he is not responding as he should be.

Most Glenn patients extubate in the OR or shortly thereafter and extubate to minimal respiratory support. Job is three days post-op and is ventilated and on a great deal of respiratory support. Why?

We're hopeful that he just needs more time to recover from surgery and his heart function will continue to improve and that he will get off the vent soon and his sats will improve. It's possible that time is all he needs. But the longer he takes the more concerned everyone gets and soon we need to explore what else it could be.

Job's Sunday morning echo might have shown problems with his aortic arch repair. He's getting another echo tomorrow morning and the results of the echo will probably tell us a lot about what the rest of this week will look like. A cardiac cath is a very likely possibility in the next few days to further explore Job's heart function if he doesn't improve in the next 24ish hours.

Job did extubate today but after about an hour needed reintubation. We think that his extubation failure was mostly a sedation problem rather than a heart problem or a lung problem. Job is in so much pain from his headaches and so uncomfortable from the breathing tube that he needs a lot of sedation. But he needs to be "awake" in order to extubate and breathe on his own. But he's so delirious from the balancing of his pain meds and sedation that he can't wake up. It's a hard balance to find. So he's off propful again and on dilaudid and we'll try again possibly tomorrow.

Job's left lung collapsed during extubation, likely because of the pressure change from invasive-ventilation (what he's had - the nasal breathing tube) to non-invastive ventilation (no breathing tube but very high support, higher even than hi-flow).

During his brief extubation!

While extubated Job had some secretions and he wasn't awake enough to cough them out, even when they suctioned, because his sedation was so deep (but again, it needs to be deep because he is so incredibly irritated and therefore agitated when he wakes up). If he had been able to cough, he probably could have kept his lung from collapsing and therefore from reintubation.

While reintubating, he got "bagged" and this popped open the top of his left lung. Overnight we'll diurese the bottom of his lung open in addition to his ventilator setting tailored to give him extra pressure support to pop it open.

The boys got to come visit today, which was really, really nice. They did not want to go to co-op this morning but did such an amazing job of being cheerful and obedient and joyfully attentive once there. I thought I couldn't be more proud of them or more grateful, but then they got to the hospital and had to spend three hours sitting in the back of Job's room basically alone, drawing and watching a TV show while I tried to help Job calm down during and after his extubation trial. That wasn't the plan for the day at all! We were going to play together and talk to Job! But they instead sacrificed their desires and served their baby brother and I.

When Job calmed down (well, was heavily sedated and muscle relaxed, following intubation) and Daddy came, they got to talk to him and it just amazes me how comfortable they are looking at his poor disfigured, swollen, scarred body. They love him so very much and are so thrilled to see him, every time. I don't know how, but they just don't even notice his incision or tubes and wires. In fact, they even call him "the cutest baby". It's hard enough for me to look at Job right now and it's such a blessing to me to watch them interact with him here post-op so fearlessly and with such adoration. I have been so concerned about how they would do through this trial over the last seven months but they have been so resilient - it is such an answer to prayer. They help me so much physically and emotionally.

All five of us!

Please keep praying for us. People remark on our strength often but we have nothing in and of ourselves. Your prayers are, through God's strength, sustaining us. We feel very weak right now. This is scary and sad. It's going to be hard waiting for answers these next few days.

Afternoon Update (9/17/16)

Job didn't have the post-op recovery nor boring night that we had hoped for.

He came out of surgery breathing on his own on the spontaneous setting on the ventilator. This setting allowed him to do most of the breathing work on his own, even though he was also at 100% oxygen (whereas room air is considered 21%).

Sadly, his sats, never as high as we wanted to begin with (well, specifically after surgery) dropped dramatically into the 50s in the middle of the night, proving he wasn't ready to breathe on his own.

The most acute patients are in rooms directly outside the attending's office. And Job had a steady stream of medical personnel in his room all night.

After his desat, his team decided to take him off of the spontaneous setting on the ventilator and increase his vecuronium (muscle relaxant) significantly, as well as his dilaudid (narcotic).

Job's heart function following surgery was and is still depressed (so he's on milrinone and was on epinephrine to help his "squeeze") and last night he didn't have the ability to work as hard as he needed to in order to maintain his sats. The idea was that if completely muscle relaxed (so his body isn't "wasting" energy), with the ventilator turned on to full capacity, Job could rest and recover a bit more and maybe his sats could recover too. And as of this afternoon it seems to have worked!

You and I only have to get used to switching our circulation once: from fetal to, well, typical. But Job will have to adjust to five different circulations: fetal to typical, typical to Norwood, Norwood to Glenn and someday Glenn to Fontan.

This is a very big adjustment for him and his little body was struggling to keep up with the work of breathing on depressed heart function, as mentioned, in addition to the normal complications of recovering from the trauma of surgery.

One such issue is his positive fluid balance. His little lungs can't function correctly with the excess fluid in them, so he needs help getting rid of that fluid through diuretics, namely lasix (item one). We need Job to have a negative fluid balance (that is, we need him to pee!) before we can move forward.

Job's hematocrit (concentration of red blood cells) was low as of this morning and so he received a transfusion (item two). In very basic terms, this means he received more red blood cells. This helps his capillary leaks heal (which helps his fluid balance) and it gives his hemoglobin a better ability to carry more oxygen around to his tissues. This made a big difference for him as he recovered from rhino a few weeks ago and the mention of it this morning made me hopeful for a similar result. I was afraid to get too excited, because of course recovering from rhino and from surgery are very different animals, but the crit infusion seems have been one of several factors in Job's turn around. SV kids (single ventricle) often respond well to crit infusions. In Job's case, it likely helped him come off of his blood pressure meds (vaso and epi).

As mentioned, in the middle of the night, Job's hard work caught up to him and he needed to do less work. His vecuronium (muscle relaxant) gave him that break, but now that he's a few more hours post-op he will be weened off of his vec so that his body can begin to breathe on his own again (item three).

I mentioned Job's new Glenn circulation and the big change that it is for his body (but I really do need to write out WHAT the Glenn is... Maybe tomorrow's project?) but in contrast to any other cardiac surgery, it is important for Glenn patients to get off the ventilator as quickly as possible. This is because the Glenn now sends passive blood flow to Job's lungs. And his lungs need the negative pressure of his diaphragm (or in his case, only the right side of his diaphragm) pulling his lungs down to better receive his passive flow. The positive pressure of the vent, pushing air into his lungs, makes it too hard for the blood to optimally drain through Glenn (that is, from his SVC to PA). The ventilator, however, provides positive pressure on his lungs and therefore Glenn patients do much better extubated (item four).

And of course, we want Job's blood pressure to remain stable as this is a primary indication of his heart function (item five). Stable blood pressure helps his profusion too, bumping his sats higher.

Surgery Day!! (9/16/16)

Today we send our baby off for his second open heart surgery (the Glenn).

Updates:

7:06 am
Here we go! Last consents and instructions! Goodbye kisses too.

8:36 am
Just got a page (I guess I fell asleep, sitting here in his empty room, waiting patiently-ish). They have now opened up Job's chest.

It will likely take them an hour to open his chest and clear away all the scar tissue from his last surgery before they can begin their heart repair work. Isn't that fascinating? It surprised me!

They no longer give updates throughout the surgery, but they page when they start and when they end, so we don't expect to hear anything else for a few more hours.


5:28pm
Job is out of surgery and recovering in is CICU room! He is stable and it sounds like the surgery itself went well.

It took a few hours longer than they were expecting and he has not been as immediately responsive to his repairs as they were hoping.

These next 24 hours will hopefully be "boring" as he's well sedated, but we expect tomorrow will be a difficult night for him. And then hopefully he'll be able to meet his goals in the coming weeks (it almost certainly will be a longer inpatient stay than we were wishing for).

We are so grateful and relieved that he made it through surgery! And we're glad to now look at this new stage of recovery.

The Day Before Surgery Pt. 2

While Seth worked, I had Ezra and Isaac with me today at the hospital. Job had a really hard day, medic needs-wise, and the boys had a really hard day, obedience-wise, and Seth had a really hard day, work-wise. And I got to try to juggle all of the moving parts. Even merely few hours removed from the most intense stress of the day, I'm wondering how I was able to handle all of that. I suppose the answer is, as always, "but God".

My favorite part of the whole mostly-awful days was when we all squeezed on top of each other in the recliner to watch a movie. A last "last for a while".

Our hour of cuddles notwithstanding, today was one of the most stressful and difficult days we've experienced. I am so glad it's almost over. I'm so glad for the freshness tomorrow brings.

Every aspect of today was just hard, culminating in a very sobering discussion of just how complex Job's aortic arch repair will be with his surgeon, Dr. Nuri. It sounds like Job will also have a longer recovery than we were anticipating because of this complexity.

Snuggles with my three boys!! 

Job was pushed up to first case, which means I'll walk to the OR with him around 6:30am. We are expecting about 7-8 hours for anaesthesia and surgery, so perhaps we will get to see him around 2:30pm.

The Day Before Surgery Pt. 1

Job is having a bit of a hard day. His oxygen needs have increased but he still keeps desatting lower and lower. He got so excited to see his brothers when we first got here that he dropped down to 53% oxygenation!! and struggled to come up, even on 2 lpm of oxygen support. I think he barely avoided a RRT intervention (rapid response team).

Three little boys were very happy to be reunited today! (The red blur is Job's sock-protected IV and board.)

When Job's body works harder (excited, angry, eating) his sats drop. His body can hardly profuse adequately even when he's at rest and he just doesn't have any reserve to work with at present.

I guess I have been surprised by how quickly his shunt function declines each day and even throughout the day. I am so glad he has been here all week! If need be (that is, if he keeps desatting so low and for so long or desats even lower), we will transfer to the CICU to increase his support.

We're no longer counting down the days but the hours until surgery.

23 hours to go.

Inpatient (Again)

Look where we are! The hospital! Again! River six, but a new room! Yay! Job just wanted to extend his tour of every room in the surgical unit and wanted a head start before surgery.

I'm giving up trying to get a good photo this morning...

Job was admitted yesterday with his lowest oxygen saturations yet. Sunday was a pretty rough day, with four episodes of emesis and declining sats. We deliberated about what to do, but he surprised us with an amazing night. We were hopeful he could make it through Monday, but he was satting around 68 (% of blood oxygenation) so Seth took him to the ER. We have been watching his heart function decline. His decreasing sats and increased cyanosis was expected. We just hoped he would make it through this week at home.

Job is, unexpectedly, responding to oxygen support. He needs his Glenn because his Sano shunt (placed as part of his Norwood at five days old) is too small for his now bigger body. What limited blood flow he is getting to his lungs is now being fully oxygenated and he's now satting in the high 70s with his cannula!!

We were supposed to have a 7-8hr pre-op appointment today but we're now doing all of his pre-op inpatient. We expect to meet with his surgeon today as well as a NP and anesthesiologist to give consent for his surgery.

I was really frustrated and disappointed yesterday, but today has been better. I think it will be hard to wait here in the hospital until Friday without the distractions of life at home. Job doesn't like his hospital bed either, but he looks so much better on oxygen that I'm very grateful we have that option. We had been so skeptical he'd make it through the week, but seeing him decline so sharply Sunday and Monday "helps" me be grateful for the opportunity to be here. Again.

It's amazing how quickly I forget to be grateful and instead focus on my discontentment. We have been given so very much, yet I still want more. And perhaps most of all, I want it on my timeline. This long journey with Job will, I'm sure (and I hope), be a very sanctifying one.

Making Memories

I took the boys to the fair today, to make a fun memory of an excursion together before the craziness of this coming week begins.



The boys are posing here, trying to look sad that they're in jail. (The new Pioneer Farm set up was so fun! We got to do so many of the things we've been reading about in Farmer Boy, like milk a cow and shave a shingle and churn butter!)

Seven days. SEVEN DAYS! This was Job's last Friday outside the hospital for a little while. Ahhh! I can't believe how soon his surgery is!!

School Started

School has started! It's going to be a busy year as Ezra and Isaac get used to doing their schoolwork again. Job will continue his pre-med education with, apparently, some tutoring on animals from Isaac prior to his upcoming "internship" at the hospital.

We've been working throughout the month of August with a soft start on our curriculum, but it's always a bit of a shock to get used to the new school year and I'm a bit apprehensive about the transition for Ezra and Isaac in particular.



We're homeschooling for many reasons, but our extracurriculars (like co-op and Sunday School and BSF) start on Monday. It's hard to move to new classrooms and teachers and be held to a regular schedule, but to do so with the added emotional context of your little brother in and (hopefully) recovering from surgery and your parents not totally present?

I know we'll make it through, but I don't anticipate it being easy.

Complicating everything is our roller coaster of emotions. Every morning we wake up and announce how many days are left until the Glenn (only 10!). We wonder, daily, if Job can make it 12 more days, 11 more days, 10 more days...

He needs this surgery so desperately! But taking him in and giving him up to, well, be sliced open, is a difficult task. I know that we have no choice, but it's hard. Really hard some days, some hours.

Otolaryngology Appointment

Job had his oto follow up today to check up on his left vocal cord paralysis. It looks as if there is slight movement! Yay! Since he eats well and cries loudly and vocalizes, they think his paralysis is
healing. He will get scoped again in the future to check for more improvement as well. He is at greater risk of aspiration with this paralysis, which is why we are monitoring closely. (And the paralysis is a result of his Norwood.)

I got to bring the big boys along and they were so obedient and helpful and quiet during the appointment!! They have (largely) been stepping up to the plate as we continue to ask hard things of them for Job's continued care. So we celebrated with pie from the cafeteria and are playing at the hospital for the day.